eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiology

Ebstein Anomaly: Differential Diagnoses & Workup

Author: Raymond T Fedderly, MD, Assistant Professor, Department of Pediatric Cardiology, Children's Hospital of Wisconsin, Medical College of Wisconsin
Contributor Information and Disclosures

Updated: Mar 9, 2009

Differential Diagnoses

Tricuspid Atresia

Other Problems to Be Considered

Uhl anomaly
Arrhythmogenic right ventricular dysplasia

Workup

Imaging Studies

  • Echocardiography is the definitive test for diagnosis of Ebstein anomaly. Typical findings include the segmental anatomy, inferior displacement of the septal and/or posterior leaflet of the tricuspid valve, large tricuspid valve annulus, dilated right atrium and right ventricle, tricuspid valve regurgitation, right ventricular outflow tract obstruction, and an atrial septal defect (ASD) or patent foramen ovale (PFO).
  • Chest radiography may be helpful in determining the size of the cardiac silhouette, which is related to the degree of tricuspid valve regurgitation. In severe cases, the cardiothoracic ratio may approach 1. A dilated right atrium is most often the cause of cardiomegaly. In patients who are cyanotic, the pulmonary vascular markings appear decreased.

Other Tests

  • Electrocardiogram: Typical findings on the ECG are large P waves, a prolonged PR interval, right axis deviation, and a right bundle branch block. Atrial or ventricular arrhythmias, as well as a delta wave indicative of Wolff-Parkinson-White syndrome (WPW) syndrome, can also be seen.
  • Holter monitor: Ambulatory ECG recording may be performed to evaluate the possibility of occult arrhythmias or to verify symptomatology, such as palpitations over 24 or 48 hours.
  • Stress test: Exercise testing may be done both preoperatively and postoperatively to objectively evaluate exercise tolerance, oxygen consumption, systemic arterial oxygenation, and possible arrhythmia vulnerability during exercise.

Procedures

  • Cardiac catheterization may be performed to evaluate both the hemodynamic and angiographic characteristics of the patient before surgical intervention. Depending on the particular patient's anatomic and physiologic findings, echocardiography findings are often sufficient, and may reduce the necessity for cardiac catheterization.
    • Postcatheterization precautions include hemorrhage, vascular disruption after balloon dilation, pain, nausea and vomiting, and arterial or venous obstruction from thrombosis or spasm.
    • Complications may include rupture of blood vessel, tachyarrhythmias, bradyarrhythmias, and vascular occlusion.
  • Electrophysiological (EP) studies are performed in many patients suspected of having an arrhythmia secondary to paroxysmal supraventricular tachycardia (SVT) and WPW syndrome, both of which have a high incidence of occurrence. During invasive EP testing, an accessory pathway is most often identified along the abnormal tricuspid annulus. Manifest accessory pathways (WPW) and concealed pathways (unidirectional retrogradely conducting accessory pathways) account for most SVT mechanistically in patients with Ebstein anomaly. The frequency of multiple accessory pathways is significantly higher in Ebstein anomaly than in the general population of patients with SVT and normal tricuspid valve anatomy.

More on Ebstein Anomaly

Overview: Ebstein Anomaly
Differential Diagnoses & Workup: Ebstein Anomaly
Treatment & Medication: Ebstein Anomaly
Follow-up: Ebstein Anomaly
Multimedia: Ebstein Anomaly
References
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References

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Further Reading

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Keywords

Ebstein anomaly, Ebstein's anomaly, Ebstein anomaly of tricuspid valve, Ebstein's anomaly of tricuspid valve, Ebstein disease, Ebstein's disease, treatment, medication, diagnosis, congenital heart defect, heart, tricuspid valve, atrial septal defect, ASD, patent foramen ovale, PFO, pulmonary stenosis, pulmonary atresia, congestive heart failure, supraventricular tachycardia, SVT, Wolff-Parkinson-White syndrome, WPW syndrome, arrhythmia, cyanosis, heart murmur, tricuspid valve regurgitation, right ventricular outflow tract obstruction

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Contributor Information and Disclosures

Author

Raymond T Fedderly, MD, Assistant Professor, Department of Pediatric Cardiology, Children's Hospital of Wisconsin, Medical College of Wisconsin
Raymond T Fedderly, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, and American Medical Association
Disclosure: Nothing to disclose.

Medical Editor

Charles I Berul, MD, Associate Professor of Pediatrics, Harvard Medical School; Senior Associate, Department of Cardiology, Children's Hospital of Boston
Charles I Berul, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, Heart Rhythm Society, and Society for Pediatric Research
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

John W Moore, MD, MPH, Professor of Clinical Pediatrics, Section of Pediatic Cardiology, Department of Pediatrics, University of California San Diego School of Medicine; Director of Cardiology, Rady Children's Hospital
John W Moore, MD, MPH is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, and Society for Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

CME Editor

Gilbert Herzberg, MD, Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College
Gilbert Herzberg, MD is a member of the following medical societies: American Academy of Pediatrics
Disclosure: Nothing to disclose.

Chief Editor

Stuart Berger, MD, Professor of Pediatrics, Division of Cardiology, Medical College of Wisconsin; Chief of Pediatric Cardiology, Medical Director of Pediatric Heart Transplant Program, Medical Director of The Heart Center, Children's Hospital of Wisconsin
Stuart Berger, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American College of Chest Physicians, American Heart Association, and Society for Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

 
 
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